Cirrhosis in Turner's syndrome: Case report and literature review

Idilman R., De Maria N., Colantoni A., Kugelmas M., Van Thiel D.

European Journal of Gastroenterology and Hepatology, cilt.12, sa.6, ss.707-709, 2000 (SCI-Expanded) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 12 Sayı: 6
  • Basım Tarihi: 2000
  • Doi Numarası: 10.1097/00042737-200012060-00023
  • Dergi Adı: European Journal of Gastroenterology and Hepatology
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.707-709
  • Anahtar Kelimeler: Chromosomal anomaly, Cirrhosis, Liver, Turner's syndrome
  • Ankara Üniversitesi Adresli: Evet

Özet

A case of cryptogenic cirrhosis in a patient with Turner's syndrome is presented. The individual was admitted for upper gastrointestinal bleeding due to oesophageal varices. After failure of medical treatment, a transjugular intra-hepatic portal systemic shunt was used to control the bleeding. A liver biopsy revealed cirrhosis with minimal necro-inflammatory activity and no steatosis. Immunohistochemical staining for HCV, HBsAg and HBcAg was negative. No other risk factor for liver disease was recognized and none of the known causes of chronic liver disease was identified after a thorough evaluation for such. Turner's syndrome is a genetic disorder due to X chromosome monosomy in which a wide range of congenital anomalies can occur. Cardiac, renal and skeletal anomalies are all well recognized. The possible association of Turner's syndrome with cirrhosis is herein discussed along with a review of the published literature. (C) 2000 Lippincott Williams and Wilkins.